Still's Disease
Adult Onset and Systemic Juvenile Idiopathic Arthritis (JIA)
Still’s Disease is a rare, multi-systemic (whole body) autoinflammatory condition that can vary from person to person. Because of a similar pathogenesis, broadly overlapping symptoms, and organ involvement, Still’s Disease represents a disease continuum that includes both Systemic Juvenile Idiopathic Arthritis (SJIA) and Adult-Onset Still’s Disease (AOSD).
Those with onset
> 16 years of age are categorized with AOSD, while those originally diagnosed with SJIA carry this diagnosis through adulthood. Onset can occur at any age, even presenting after birth. In some individuals, Still’s Disease develops suddenly, disappears almost as quickly, and may not return for many years, if ever. In others, it is a chronic condition.
In 2017, the International Still's Disease Foundation (ISDF) dissolved and, as a trusted collaborator over the years, requested that our organization house any existing resources. Read the original dissolution announcement letter.
Symptoms of Still's Disease
Still's Disease symptoms and progression vary per individual. Some patients will have many of these symptoms, some only a few. This makes detecting, diagnosing, and treating Still’s difficult. Severity and frequency will vary in degree based on the individual.
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RashList Item 1
Most commonly a salmon colored rash appearing on the upper anterior torso, arms, and legs that may quickly fade. Typically does not itch or mild itch, however patients reported a rash that can itch, be raised, appear as a number of small, flat red spots, and may not quickly fade away. May not be visible if covered with hair or if it fades very quickly. (2)
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FeverList Item 2
High and spiking, rising to 104 degrees F/41 degrees C or higher and can rapidly return to normal levels or below. Time of day can vary, but evening and nighttime reported more often. May come and go, sometimes twice a daily, but may last a week or longer. Could cause for patient-reported excessive sweating/night sweats. (3)
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FatigueList Item 3
Reported in > 90%*; common in all autoinflammatory diseases, not unique to Still’s.
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MyalgiaList Item 4
Reported in > 75%* of patients, some described similar to flu-like weakness.
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Sore Throat
All age groups, even higher reports in children. Some report difficulty swallowing.
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Inflammatory ArthritisList Item 1
Historically arthritis, or joint involvement, was considered one of the major Still's Disease symptoms. However, recent research suggests about 25% of patients may not have arthritis at or near onset - some may not develop it for years, if ever. In others, the arthritis is severe. Swelling, redness, warmth, and joint erosion is possible. Can cause severe stiffness after rest.
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Organ InvolvementList Item 2
Generalized lymph nodes/glandular swelling. Enlargement of the liver and/or spleen (4). Inflammation of lungs (pleuritis), the saclike covering of your heart (pericarditis), the muscular portion of your heart (myocarditis) (5), eyes, serous tissues (6). Organ involvement may be present at onset.
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GastrointestinalList Item 3
Nausea, weight loss, poor appetite, canker sores in mouth.
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Central Nervous System ManifestationsList Item 4
Including seizures, meningismus.
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Cognitive Dysfunction aka “Brain Fog”*
May be associated with inflammation in the brain caused by the immune response (7).
Diagnosing Still's Disease
Still’s Disease is a diagnosis of exclusion, requiring all other conditions to be ruled out first. Delayed diagnosis is common, as Still’s Disease is often confused with infection. Although current research is developing, there are no specific tests or biomarkers (9).
Common laboratory abnormalities for Still’s Disease patients include:
- Elevated erythrocyte sedimentation rate (ESR)
- Elevated leukocytes, especially neutrophils (white blood cells)
- Thrombocytosis (high platelet count)
- Elevated ferritin levels (sometimes dramatically) Negative rheumatoid factor (RF) test (10)
- Negative Antinuclear antibodies (ANA) test
- Anemia (<10 g/dl)
- Hypoalbuminemia (low albumin levels < 3 g/dl)
- Mild elevated liver enzymes
Cause of Still's Disease
Some researchers suspect the condition might be triggered by a viral or bacterial infection or genetic mutations. While Still's is an autoinflammatory disease (of the innate side of the immune system), research suggests both sides of the immune system may be triggered, causing autoimmune (of the acquired side of the immune system) features as well. Learn about the sides of the immune system.
*These statistics and experiences are the result of our internal research, then medically reviewed by the Center for Autoinflammatory Diseases. Therefore, the percentages may vary from research conducted elsewhere.
Other Complications Associated With Still's Disease
Macrophage Activation Syndrome (MAS) - a massive inflammatory response that overwhelms the whole body, including organs. MAS is more common in SJIA and may be present at onset (8). Check out our MAS page for information, resources, and patient stories.
Mental Health
Mental health - A quarter of patients surveyed reported mental health issues including: irritability, low self esteem, and difficulty coping with stress, anxiety, isolation, frustration, and depression.
Still's Disease Resources & Support
At AiArthritis, we place community voice at the heart of everything we do. Our Still’s Disease resource hub was created through collaboration with patients and clinicians to develop materials that reflect lived experiences and medical expertise. Here you can explore brochures, educational tools, awareness materials, and clinician resources designed to educate, empower, and support personalized care.
The dragon shown is the original logo designed by the International Still's Disease Foundation, who dissolved into AiArthritis in 2017 and is replicated in the Still's Disease brochures and emergency cards.
Patient-Reported Still's Disease Brochures & Emergency Cards
Our #MyStills Still’s Disease brochures and folding information cards are available as free, downloadable PDFs in English, Spanish, and French. The brochures were co-created with Still's patients and health professionals. Brochures provide a clear overview of the disease, while the compact emergency/new health professional folding cards highlight key symptoms, important testing, and space for patients to write personalized alerts.
Quick Still's Disease Symptom Sheet
This resource was developed from patient-reported disease experience research conducted by our organization - combining the results from our Early Symptoms of AiArthritis study (2013-2015) and peer-reported data.
International Still's Disease Support Center
One resource AiArthritis took over upon the dissolution of the International Still's Disease Foundation (ISDF) is the Facebook Still's Disease Support Group, where those affected by Still's can ask questions and gain support from others like them. If you are a person living with Still's (a patient or family member/significant other), join today!
Still's Disease Awareness
Still’s Disease Awareness shines a light on this rare autoinflammatory condition, helping to educate the public, accelerate diagnosis, and empower patients living with systemic juvenile idiopathic arthritis and adult-onset Still’s disease.
September 7th is Still's Disease Awareness Day
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#MyStills Awareness and Educational Posters
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#MyStills Campaign and Patient Experience Videos
By collecting these stories, we hope to create more awareness and education about Still's Disease for patients, their support network, the public, and the medical community.
References
1) Research based on 2015
Early Symptoms Study (ESS) combined with 2019-2020 focus groups and survey participation, both conducted by the International Foundation for Autoimmune & Autoinflammatory Arthritis.
2) There is increasing evidence of atypical rashes: raised, red or purple plaques; itchy; bumps that closely resemble eczema or urticaria. Atypical rashes, in conjunction with other typical Still’s symptoms, should be tested to rule out other causes, such as urticarial vasculitis.
3) Some patients reported feeling extremely hot and flushed, without registering a fever.
4) May be cause of abdominal pain.
5) May be responsible for patient-reported “shallow breathing” or “chest pain”.
6) Serositis refers to inflammation of the serous tissues of the body, the tissues lining the lungs, heart, and the inner lining of the abdomen and organs within.
7) Medical reviewers could not confirm this symptom as related directly to Still’s Disease.
8) Haemophagocytic lymphohistiocytosis (HLH) is termed macrophage activation syndrome (MAS) when associated with rheumatic disease (secondary HLH) and triggers including malignancy and infection.
9) Some studies suggest certain cytokine profiles (interleukins 1, 18 and IL18BP) and human leukocyte antigen (HLA) genotypes may provide diagnostic clues, but no consistent results are clinically validated.
10) Rheumatoid factor can be positive in healthy people, so having a positive RF should not dictate diagnosis.
